Coughing, wheezing, breathlessness during exercise, and increased sputum production are typical cystic fibrosis symptoms. However, these are also the symptoms of a complication that affects cystic fibrosis patients, termed allergic bronchopulmonary aspergillosis (ABPA). According to the Cystic Fibrosis Foundation, children affected by ABPA are allergic to the Aspergillus bacteria that colonizes their lungs, leading to worse flare ups than those seen in cystic fibrosis patients without ABPA. Since ABPA is a serious complication, researchers in the Cystic Fibrosis Center at the Institute of Mother and Child in Poland were interested in determining the prevalence and predispositions for ABPA in children with cystic fibrosis.
Dr. K. Walicka-Serzysko and Dr. D. Sands wrote about their findings in the article “The Clinical Presentations of Pulmonary Aspergillosis in Children with Cystic Fibrosis – Preliminary Report,” which was published in the journal Developmental Period Medicine. Over four years of data collected from 374 patients ages 0 to 18 years were compiled for the team’s study. Thirteen of these patients had especially threatening clinical status and decline in lung function despite standard treatment for cystic fibrosis. All thirteen showed signs of Aspergillus fumigatus in their sputum, with three being diagnosed with ABPA and the rest being diagnosed with Aspergillus colonization or Aspergillus infection.
All ABPA patients showed pancreatic insufficiency, and most also had chronic respiratory colonization with Staphylococcus aureus and Pseudomonas aeruginosa, in addition to Aspergillus fumigatus. To combat these infections, all ABPA patients were placed on a long-term oral azithromycin regime. Some patients with Aspergillus infection received lung surgery, with one boy receiving a lung transplant nine months after diagnosis with Aspergillus infection.
The goal of collecting this data was to identify the risk factors for developing ABPA. However, too few patients were identified in the study to make significant relationships between ABPA and any prominent clinical features. The researchers were also relying on retrospectively compiled data and did not initiate a prospective study with the specific intention of following cystic fibrosis patients over time to evaluate any risk factors for ABPA.
While data from the study reveals the overall risk for developing ABPA in cystic fibrosis is low (0.8%), the study authors note that annual screening for CF patients 6 years and older for early detection of Aspergillus colonization is recommended, since pulmonary aspergillosis is a very serious complication in CF children. The researchers also feel that, due to the limitations of the study design, further prospective studies are needed to help identify additional pulmonary aspergillosis risk factors in pediatric CF patients.